OC13\u2005Sydenham’s chorea – A rare condition?

Abstract

Sydenham’s chorea (SC) is considered a ‘rare disease’ in Western Europe and not much is known about its incidence in the UK 1.SC is a major criteria for diagnosing Acute Rheumatic fever (ARF). It is known to affect 10–30% of children with ARF. Carditis can affect 50–70% of children with ARF3. Damage to cardiac valves in ARF could be chronic and progressive. This is an abstract of two patients presenting to A&E with involuntary movements. Both had chorea, carditis and significantly raised ASO titres and were diagnosed to have ARF based on modified Jones criteria. Case 1 A 9 year old boy attended A&E with 2 weeks history of being increasingly clumsy and fidgety. Had repetitive involuntary movements of arms and multiple facial grimaces. He developed change in speech and was noted to get agitated easily. He had tonsillitis 2–3 weeks before, managed as viral illness by GP. Examination findings were consistent with SC; He was diagnosed with carditis a week later, with echocardiogram evidence of mild mitral and aortic valve regurgitations. His initial ASO titre was 2288, increased to 2626 at 6 weeks and decreased to 1489 11 weeks post presentation. He was commenced on sodium valproate 10 mg/kg twice daily 6 weeks later due to worsening chorea. 6 weeks following treatment, parents reported marked symptomatic improvement. Case 2 A 9 year old girl presented with six day history of being fidgety, having abnormal movements in arms and poor balance. Child had fever, cough, and hoarse voice 2 months previously and was treated as viral illness. Examination findings were consistent with SC. Her ASO titre was 481. Echocardiography showed mild mitral and tricuspid regurgitation. She was commenced on Haloperidol, symptoms improved completely at 6 months and dose was gradually reduced and stopped. Both patients had negative throat swab culture and normal cranial MRI. Lactate, Thyroid functions, Very long chain fatty acids, NMDA receptor antibodies, ANA titres, plasma amino acids, Copper, ceruloplasmin levels, Urine organic and amino acids were all normal. Both patients were started on Penicillin prophylaxis. Summary We encourage paediatricians to consider SC as a diagnostic possibility in children presenting with involuntary movements and emphasise importance of cardiac evaluation in them. We also believe there is a possible increase in incidence of SC and hence a need to develop consensus guideline regarding medications for SC. We highlight use of either Valproic acid or Haloperidol in SC with good effects.